Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia.

DESCRIPTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications. METHODS: The guidelines were developed using the AGREE II (Appraisal of Guidelines for Research and Evaluation II) framework and GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. The guidelines expert panel included expert physicians (clinical and genetic) in HHT from 15 countries, guidelines methodologists, health care workers, health care administrators, patient advocacy representatives, and persons with HHT. During the preconference process, the expert panel generated clinically relevant questions in 6 priority topic areas. A systematic literature search was done in June 2019, and articles meeting a priori criteria were included to generate evidence tables, which were used as the basis for recommendation development. The expert panel subsequently convened during a guidelines conference to conduct a structured consensus process, during which recommendations reaching at least 80% consensus were discussed and approved. RECOMMENDATIONS: The expert panel generated and approved 6 new recommendations for each of the following 6 priority topic areas: epistaxis, gastrointestinal bleeding, anemia and iron deficiency, liver VMs, pediatric care, and pregnancy and delivery (36 total). The recommendations highlight new evidence in existing topics from the first International HHT Guidelines and provide guidance in 3 new areas: anemia, pediatrics, and pregnancy and delivery. These recommendations should facilitate implementation of key components of HHT care into clinical practice.

The Accuracy of Acetabular Cup Positioning Using Patient-Specific 3D Orientation Guidance in Total Hip Arthroplasty.

INTRODUCTION: Malpositioning of the acetabular cup during total hip arthroplasty (THA) increases the risk of certain complications and shortens the lifespan of the prosthetic joint. Therefore, the accurate placement of the acetabular component during a THA is a necessary contributing factor to its successful outcome. The different methods of intraoperative estimation of acetabular component positioning are quite varied and sometimes may be inaccurate. The purpose of this study was to assess the accuracy of intraoperative acetabular component orientation with the assistance of three-dimensional (3D), patient-specific guidance alone. MATERIALS AND METHODS: At a single institution, a total of 56 patients were prospectively enrolled into this study. Acetabular cup positioning was achieved with a described method using a laser beam technique minus the placement of pelvic pins. Comparison was made between the planned, preoperative inclination and version angles with the achieved postoperative inclination and version of the acetabular component in all THAs performed. The accuracy of placement of the acetabular cup was assessed using postoperative computed tomography (CT) scans. Evaluation was performed by an independent orthopaedic surgeon. RESULTS: Fifty-eight hips were included in the present study. The mean absolute deviation from the preoperative planned inclination and anteversion was 4.0° (0.1° to 14.6°; p<0.05) and 4.4° (0.2° to 12.2°; p<0.05) respectively. The planned inclination and anteversion were achieved within a +/- 10° target in 98% of the cases respectively. CONCLUSION: Accuracy of acetabular cup orientation in total hip arthroplasty can be achieved to a high degree with 3D patient-specific guidance alone. This eliminates the pins in the pelvis and has the potential to reduce costs and patient trauma without impacting accuracy.

Reliability of the transverse acetabular ligament as a landmark for functional cup anteversion in total hip arthroplasty.

BACKGROUND: The transverse acetabular ligament (TAL) can be a useful and reproducible landmark in the orientation of the acetabular cup in total hip arthroplasty (THA). Its role in guiding cup orientation when aiming to implant in a functional anteversion orientation is unclear. The aim of this study was to assess the relationship between the TAL and the planned acetabular cup anteversion when implanted in a function orientation. MATERIAL AND METHODS: In a retrospective study the anteversion of the TAL in the contralateral un-replaced hip was measured in CT scans of patients undergoing THA and compared to the functional cup anteversion using the patient-specific spinopelvic parameters. Comparative measurements of the native acetabular version were made from the superior rim to the inferior rim and at 10 mm intervals between the 2, all in reference to the anterior pelvic plane. RESULTS: 96 hips were measured. The mean TAL anteversion angle was 17.2° ± 4.5°. The mean planned acetabular cup anteversion angle was 26.3° ± 4.7°. Pearson's correlation coefficient of this measurement with the TAL was -0.03 (p = 0.769). There was a significant difference between the planned acetabular cup anteversion and the measurement of the TAL (p < 0.0001). CONCLUSIONS: If cups are implanted parallel to the TAL, almost 80% will be >5° different to targeted functional cup version. It should be aimed to implant cups with more anteversion than the TAL indicates.